Chest
Volume 99, Issue 6, June 1991, Pages 1485-1494
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Critical Care
Journal Article
The Diagnosis and Management of Neuromuscular Diseases Causing Respiratory Failure

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The Neuroanatomy of Respiration

The brain stem is the primary center for the central control of respiration. This control occurs at a subconscious level and results in the rhythmic contraction and relaxation of the respiratory muscles. This automatic state can be temporarily overridden by voluntary mechanisms or by reflex actions such as coughing or sneezing.2 These voluntary mechanisms are essential for speech and phonation. The cortical centers for the voluntary control of respiration are presently not well localized.

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An Anatomic Approach to the Diagnosis of Neuromuscular Diseases

Figure 1 demonstrates the neuroanatomy of the respiratory muscles. Only lesions along the pathways demonstrated in this figure can produce weakness of the respiratory muscles. Diagnosis of the cause of neuromuscular dysfunction is best made by anatomically localizing the site of the lesion by history and physical examination. Once the anatomic site (ie, myoneural junction, spinal cord) is determined, the correct diagnosis can be confirmed by associated nonneurologic symptoms and other tests.

Central Disorders

Stroke is a common cause of upper motor neuron respiratory dysfunction. Strokes occur most frequently in the middle cerebral artery distribution11 and affect the voluntary system of respiration. Strokes can alter respiratory function in several ways. First, they often affect the muscles that protect the upper airway and maintain its patency. Horner et al12 found that among 47 patients with stroke, one half showed signs of aspiration. Although patients with brain-stem strokes with resultant

Precipitating Factors

Precipitating factors are often the immediate cause for ICU admission of patients with neuromuscular disease. The identification of such factors is essential because they may be more amenable to therapy than the neuromuscular disease itself. Upper airway obstruction and aspiration should be suspected in patients with bulbar dysfunction, whereas microatelectasis and lower respiratory tract infections are common among all patients with generalized weakness. Pulmonary hypertension and right-sided

ACKNOWLEDGMENT

The authors thank Dr. June Melloni-Kelly for her superb illustration. Thanks are also extended to Dr. Neal H. Cohen and Dr. Anthony Campagna for their critical review of the manuscript.

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